The variant NM_004086.3:c.260G>T in COCH is a missense variant predicted to cause substitution of Glycine to Valine at amino acid 87 (p.Gly87Val). This variant was not found in gnomAD v4.1.1 (PM2_Supporting met). The in silico predictor REVEL yields a score of 0.867 (PP3 met). This variant was identified in two probands (PS4_Supporting met), including one from an East Asian family reported in two publications (HL-VCEP internal data, PMID:23993205, 23767834), in which it segregated with autosomal dominant, progressive, late-onset HL and concomitant vestibular dysfunction in 4 affected family members (PP1_Moderate met). Given the highly specific phenotype of autosomal dominant progressive HL with vestibular dysfunction, the HL-VCEP decided to apply PP4 evidence criteria. A different missense variant c.259G>T (p.Gly87Trp) at the same amino acid position has been classified as pathogenic by the ClinGen HL-VCEP (PM5 met). In summary, based on the current evidence (PM5, PP1_Moderate, PS4_Supporting, PP4, PP3 & PM2_Supporting), the variant NM_004086.3(COCH):c.260G>T (p.Gly87Val) is classified as likely pathogenic for autosomal dominant non-syndromic SNHL based on the ACMG/AMP criteria applied, as specified by the ClinGen HL-VCEP (ClinGen Hearing Loss VCEP specifications version 2; 09/16/2026).